Granulomatosis with polyangiitis: An atypical initial presentation.

Granulomatosis with polyangiitis (GPA) is a necrotizing vasculitis of small and medium vessels with involvement of the upper and lower respiratory tract and necrotizing pauci-immune glomerulonephritis [1]. This vasculitis has a higher incidence in men in the sixth decade of life and more than 80% of patients have positive anti-neutrophil cytoplasm (ANCA) antibodies [1,2]. We present the case of a 23-year-old man with two weeks of evolution with polyarthralgia, asthenia, and cough with hemoptoic sputum. He did a chest radiography that showed diffuse bilateral alveolar infiltrates, on the second stage. The patient presented a rapid clinical worsening, with moderate hemoptysis and severe respiratory failure requiring invasive mechanical ventilation. The autoimmune study revealed positivity for ANCA PR3 in titer >200, having started pulses of methylprednisolone, plasmapheresis and later cyclophosphamide, with clinical improvement. His high-resolution chest computed tomography (CT) showed areas of diffuse ground glass densification suggesting capillaritis/alveolar hemorrhage and two subpleural nodular areas suggestive of granulomatous vasculitis. CT of the nasal sinuses showing findings compatible with acute inflammatory changes, with histology of the nasal mucosa inconclusive. Thus, this case shows an exuberant and potentially fatal form of diffuse alveolar hemorrhage that culminated in the initial diagnosis of granulomatous vasculitis in a young adult.

Deep Venous Thrombosis and Pulmonary Thromboembolism Associated With Retroperitoneal Hematoma in a Patient With Ehlers-Danlos Syndrome Type VI.

Ehlers-Danlos Syndrome (EDS) is a group of genetic diseases of the connective tissue, which is rare and is characterized by joint hypermobility, tissue, and vascular fragility. We present the case of a 38-year-old woman with a known diagnosis of EDS type VI who came to the emergency room, complaining of sudden dyspnea in the context of abdominal pain and pain in the left lower limb with one week of evolution. Computed axial tomography showed the presence of bilateral pulmonary thromboembolism, iliofemoral thrombosis, and a retroperitoneal hematoma. Anticoagulation was started and the patient was admitted to the intermediate care ward. This case highlights the need to know the implications of increased vascular fragility in type VI EDS, with potentially serious consequences. Underlying is the inevitable need for judicious consideration of the decision to undergo anticoagulation, in the context of a retroperitoneal hemorrhagic event and pulmonary thromboembolism.